Maxillofacial MNTI: Diagnostic And Surgical Insights From Six Infant Cases

Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare neoplasm in early infancy, particularly in the maxillofacial region. It is characterized by rapid growth and significant deformation. Management of MNTI remains controversial due to the lack of standardized therapeutic protocols. This study analyzes six maxillary MNTI cases to improve early diagnostic accuracy and refine surgical strategies. Six infants (1-8 months old) with MNTI (2016-2023) presented with rapidly expanding masses. These included gray-black pigmented lesions with intact overlying mucosa and palpable firm masses. CT imaging revealed combined osteolytic and sclerotic bone changes. Four cases were initially misdiagnosed. All underwent surgical treatment, during the follow-up, two cases recurred: one 1 month postoperatively, which was re-excised with extended margins and remained recurrence-free for 5 years; the other recurred 5 years later, showing malignant transformation and requiring extended resection, chemotherapy, and radiotherapy. MNTI has typical clinical and radiological features, it can help to diagnose earlier. During surgery, while soft tissue can be remove completely, bone involvement is challenging. Careful preoperative review of X-rays and endoscopic assistance can help achieve near-complete resection. Follow-up showed that some cases with incomplete resection did not recur, but one case transformed malignantly 5 years later, highlighting the need for long-term follow-up. Early diagnosis of MNTI can be achieved by recognizing its typical clinical manifestations. Complete surgical resection is effective. The lesion has the potential for malignant transformation, emphasizing the necessity of regular follow-up.